1. The Registry. European Cystic Fibrosis Society. Updated February 13, 2019. Accessed December 7, 2022. https://www.ecfs.eu/projects/e... Google Scholar

Liver changes observed in the course of cystic fibrosis comprise a group of complex processes of fibrosis, inflammation, remodelling, apoptosis and cholestasis as a result of abnormal functioning of the cystic fibrosis transmembrane conductance regulator (CFTR) protein, immunological reactions and response to oxidation stress. Liver lesions are only observed in 5-20% of patients with diagnosed ...

Cystic fibrosis is a common genetic disorder characterized by a severe lung inflammation and fibrosis leading to the patient's death. Enhanced angiogenesis in cystic fibrosis (CF) tissue has been suggested, probably caused by the process of inflammation, as similarly described in asthma and chronic bronchitis. The present study demonstrates an intrinsic pro-angiogenic status of cystic fibrosis ...

Abstract Respiratory infections caused by non‐tuberculous mycobacteria (NTM) are a major cause of morbidity for patients living with cystic fibrosis (CF), as NTM pulmonary disease (NTM‐PD) is challenging to both diagnose and eradicate. Despite the lengthy courses established regimens recommended Cystic Fibrosis Foundation (CFF) European Society (ECFS) consensus guidelines, only about 50% 60% ac...

In recent years, the microbiome has been recognized as a key regulator of immune responses. Evidence suggests that changes in the microbiome can lead to chronic disease and even exacerbation of the disease. Impairment of innate immunity resulting from microbial incompatibility may worsen host susceptibility to infection and exacerbate chronic lung diseases. Specific microbes play a key role in ...

The principal clinical manifestations of cystic fibrosis are bronchiectasis with chronic pulmonary infection and pancreatic malabsorption due to destruction of the exocrine pancreas.' The interactions between the lung disease and nutrition are important because nutritional state usually parallels declining lung function and is a predictor of survival.2 A better understanding of how these two fa...